Fig. 7.

Sphingosine in tissues of Farber disease mice receiving repeat injections of rhAC. Approximately 3-week-old Farber disease mice received once weekly i.p. injections of purified rhAC at doses of 1 mg/kg, 3 mg/kg and 10 mg/kg (n = 4–10/dose). Mice were euthanized when they lost > 10% body weight within a one week period. Total sphingosine (Sph) levels were determined in tissue extracts as described in the Materials and Methods. “0” indicates Farber disease mice receiving once weekly injections of PBS. *indicates p value < 0.05 comparing mice treated with rhAC to mice treated with PBS. **indicates p value < 0.01.